A case of congenital absence of the nose is presented. The etiology of this rare condition is unknown. A review of the literature reveals that the previously applied terms, e.g. 'arhinia', are unclear. In the reviewed cases there seems to be a pattern of facial anomalies associated with nasal absence. In most cases, one could probably expect a lack of the olfactory bulbs and tracts. We suggest a new terminology and summarize the aims of the radiological evaluation of this condition.